Coenzyme Q (CoQ) is part of the cellular membranes in all aerobic organisms. CoQ is synthesized in the mitochondrial matrix by a multiprotein complex. In the mitochondria, CoQ is instrumental for mitochondrial electron transport transferring electrons from complexes I and II to complex III. CoQ is essential for pyrimidine nucleotide biosynthesis and oxidation of fatty acids. During these processes, the redox state of CoQ determines the leak of electrons and the subsequent generation of mitochondrial Reactive Oxygen Species (ROS) and oxidative damage. In addition, in the plasma membrane, CoQ also participates in electron transfer reactions carried out by enzymes such as cytochrome b5 reductase or NQO1 whose expression has been shown to impact the rate of ageing. Being such an important molecule is not surprising that mutations in genes involved in CoQ metabolism have been reported in multiple patients suffering mitochondrial diseases. Moreover, alterations in CoQ metabolism have been reported in ageing including cardiovascular ageing, where CoQ supplementation protects against the side effects of statins. In this Research Topic, we will discuss the former topics in detail, putting special attention to the role CoQ has in ageing and the pathogenic mechanisms underlying CoQ deficiencies.
Coenzyme Q (CoQ) is part of the cellular membranes in all aerobic organisms. CoQ is synthesized in the mitochondrial matrix by a multiprotein complex. In the mitochondria, CoQ is instrumental for mitochondrial electron transport transferring electrons from complexes I and II to complex III. CoQ is essential for pyrimidine nucleotide biosynthesis and oxidation of fatty acids. During these processes, the redox state of CoQ determines the leak of electrons and the subsequent generation of mitochondrial Reactive Oxygen Species (ROS) and oxidative damage. In addition, in the plasma membrane, CoQ also participates in electron transfer reactions carried out by enzymes such as cytochrome b5 reductase or NQO1 whose expression has been shown to impact the rate of ageing. Being such an important molecule is not surprising that mutations in genes involved in CoQ metabolism have been reported in multiple patients suffering mitochondrial diseases. Moreover, alterations in CoQ metabolism have been reported in ageing including cardiovascular ageing, where CoQ supplementation protects against the side effects of statins. In this Research Topic, we will discuss the former topics in detail, putting special attention to the role CoQ has in ageing and the pathogenic mechanisms underlying CoQ deficiencies.
